급성 간헐성 포르피리아에 대한 임상적 고찰

김광수,유경무,유영수 고신대학교 의학부 1996 高神大學校 醫學部 論文集 Vol.11 No.1-2

1988년 1월부터 1995년 12월까지 고신대학교 복음병원과 계명대학교 동산병원에서 급성 간헐성 포르피리아(acute intermittent porphyria, AIP)로 진단된 환자 17례를 조사대상으로 하여 임상관찰 후 다음과 같은 결과를 얻었다. 1) 발병연령은 10-59세(평균 25.8±14.1세)로서 10대가 전체의 47%로 가장 많았으며, 성별분포는 여자가 12례(70.6%)로 남자보다 많았다. 2) 가족력은 전체 17례 중 2례(11.8%)에서 있었다. 3) 발병촉진인자는 상기도감염이 5례(29.4%)로 가장 흔하였으며, 알코올섭취, 월경, 절식, 및 스테로이드복용이 각각 1례였다. 4) 임상증상으로 복통(100%), 변비(47%), 오심구토(47%) 등이 흔하였으며, 임상징후로는 빈맥(82.3%), 발열(52.9%), 고혈압(47%) 등이 흔하였다. 그리고 발작은 29.4%에서 관찰되었다. 5) 말초혈액검사에서 백혈구증가증이 3례(17.6%), 빈혈이 6례(35.3%)에서 관찰되었으며, 요검사에서는 우로빌리노겐 요증이 전례, 단백뇨 5례(29.4%), 당뇨 4례(23.5%), 및 빌리루빈뇨 4례 등이었다. 혈액화학검사에서는 GOT증가 6례(35.3%), GPT증가 5례(29.4%), 및 총단백량감소 2례 등이었으며, 저나트륨혈증이 6례(35.3%) 및 저칼륨혈증이 3례(17.6%)였다. 뇌척수액검사는 1례에서 단백이 약간 증가되었으며 그 외에는 정상이었다. 6) 포도당 정맥주사로 치료받은 대부분 환자에서 예후는 양호하였다. Acute intermittent porphyria(AIP) is a rare inborn error of metabolism characterized biochemically by the hyperexcretion of the porphyrin precursors δ-aminolevulinic acid(ALA) and porphobilinogen(PBG) in the urine, and clinically by widespread lesions in the nervous system. The clinical manifestations were observed in 17 patients who were diagnosed as AIP at Kosin University Gospel Hospital and Keimyung University Dongsan Hospital between Jan 1988 and Dec 1995. The results were summarized as follows : 1) The age at onset were 10-59 year old (mean 25.8±14.1 year old). The prevalence in female is higher. 2) The family history was observed in 11.8% of cases. 3) The most common precipitating factor was upper respiratory tract infection. 4) Majority of patients showed abdominal pain, tachycardia, fever, hypertension, constipation, nausea and vomiting. 5) Conventional laboratory findings were nonspecific. 6) The prognosis was good to excellent in the properly treated cases with intravenous administration of glucose.

원발성 여성 요도 종양의 임상특성 및 추적관찰

정창호,류현열 고신대학교 의학부 2004 高神大學校 醫學部 論文集 Vol.19 No.1

Background : Female urethral lesions encompass a spectrum of entities that have significance in clinical urology. With that, female urethral lesions have rarely been reported and the differential diagnosis between benign and malignant lesions has not been established. I investigate retrospectively the incidence and the clinical presentations of female urethral lesions by my experience about 20 cases of the female primary urethral tumor. I also analyze the data to determine the optimum procedure for diagnosis and therapy. Method : The study included 20 patients with female urethral tumor treated by surgical procedure at Masan Samsung hospital of Sungkyunkwan University between Jan. 1994 to Apr. 2001. We investigated the clinical aspect of the female urethral tumor by symptom of each lesion, the spread of the patient's age, the method of the diagnosis and treatment. The preoperative evaluation included a thorough clinical history, physical examination, urinalysis, urine cytology, cystourethroscopy, and the radiologic studies, including voiding cystourethrography, retrograde urethrography, pelvic ultrasonography, pelvic CT or MRI. Results : The age range of the patients, who were surveyed for the 20 cases of the primary tumor, was between 25.2 and 74.6 (mean age 46.9 years), and 8 of them were in the period of menopause. We can inspect urethral diverticulum and we can treated it through diverticulectomy only. 4 cases were necessary to use Martius interposition graft when the path between urethra and the diverticulum was large. 2 cases had the stress incontinence postoperatively and there were necessary anterior vaginal wall sling procedure. The tumor was removed under transvaginal approach in urethral leiomyoma and leiomysarcoma and had not seen a significant morbidity. Urethral transitional cell carcinoma was found that it metastasized to the inguinal lymph nodes and distant metastasis to lung and bone after radical total cystourethrectomy. After the surgical excision of the benign urethral lesions, there would be no relapse or progression to malignancy. Conclusion : Urethral masses are essentially needed radiologic or endourologic procedure and pathologic examination due to possible development of a malignant tumor even though it is rare. A large scale of the urethral tumor like the leiomyoma of urethral diverticulum could be cured and safely managed by surgical excision, but the preventive procedure of the stress incontinence was needed. Finally it is important to perform auxiliary radiotherapy or chemotherapy with radical cystourethrectomy in female urethral malignant tumor to prevent the disease progression.

폐경전후 및 폐경후 골감소에서 호르몬대체요법과 Fluocalcic Effervescent® 병합치료 후 골밀도 및 골대사의 생화학 지표의 변화

어완규,이창원 고신대학교 의학부 2004 高神大學校 醫學部 論文集 Vol.19 No.1

Background: Sodium fluoride stimulates bone formation and has been used to treat osteoporosis for decades despite debate about the antifracture efficacy. For women with low bone mass, the ideal therapy should not only inhibit bone resorption but simultaneously stimulate bone formation to increase the bone mass, the ideal therapy should not only inhibit bone resorption but simultaneously stimulate bone formation to increase the bone mass above the fracture threshold. We thus performed an intervention study to investigate the effect of a low dose fluoride prospectively, in combination with HRT, on bone mineral density (BMD) and biochemical markers of bone turnover. Methods: Thirty perimenopausal or postmenopausal osteopenic women were enrolled, and were treated with Fluocalcic effervescent® (disodium monofluorophophate [MFP] 100㎎ + calcium carbonate 1,250 ㎎) 1 tablet BID and conjugated equine estrogen (HRT) 0.625㎎ QD for 12 months in Department of Internal Medicine, Kosin University Gospel Hospital from January 2001 to December 2002. BMD and chemical markers of bone turnover, serum alkaline phosphatase, serum osteocalcin, and urine deoxypyridinoline(DPD), were evaluated before and 12 months after starting treatment. Results: Serum alkaline phosphatase, serum osteocalcin, and urine DPD after 12 months were not significantly different from those of pre-treatment values. The post-treatment BMD's but they were not correlated with all the pretreatment chemical markers of bone metabolism. Changes of the BMD of L2-L4 were not correlated with the changes of chemical markers except urine DPD. Pretreatment markers, BMD of L2-L4 , serum osteocalcin, serum alkaline phosphatase, and FSH were correlated with changes in BMD of L2-L4. There was no chemical markers correlated with the change in BMD of femoral neck. Conclusion: Twelve-month treatment with MFP and HRT was not associated with improvement in BMD. Pretreatment markers, BMD of L2-L4, serum osteocalcin, serum alkaline phosphatase, and FSH may predict changes in the BMD of L2-L4.

복수를 동반한 호산구성 장염 2례

김신,김유리,하승인,이지현,임현정,박무인,박선자,구자영,이창연 고신대학교 의학부 2004 高神大學校 醫學部 論文集 Vol.19 No.1

저자들은 복수가 생긴 다양한 소화기 증상을 나타내는 2명의 환자들에게서 복수와 말초혈액검사에서 호산구증가를 통해서 호산구성 위장관염을 진단하고 스테로이드를 사용 후 호전을 보인 2예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Eosinophilic gastroenteritis is a rare condition of unknown cause characterized by peripheral eosinophilia. eosinophilic inflammation and various gastrointestinal symptom. Eosinophilic gastroenteritis is generally classified into three types according to the layer of gastrointestinal tract involved(mucosal type, muscularis type, serosal type) and clinical manifestation are related to the site of involved gastrointestinal tract. Especially, serosal type is rarest form of them and occasionaly associate with asictes. We report 2 cases of eosinophilic gastroenteritis with ascites.

화학적 손상에 따른 간 재생의 '간 조상세포' 동태에 관한 연구

허방,박명선,윤명희,이충한 고신대학교 의학부 2004 高神大學校 醫學部 論文集 Vol.19 No.1

Background: Oval(OV) cells had been described as an uncertain type of cells in early neoplastic process of animal hepatocarcinogenesis model. Although biological significance of OV cells is not elucidated in human, they showed cholangiolar and hepatocytic bipotential differentiation in animals, especially rodents. With recent studies emphasizing on a possible target of gene therapy and cell transplantation of hepatic stem cells, biological investigation of OV cells become to be an important issue. In present study, the authors pursue the fate of hepatic stem cell progenitor cells such as OV cells using 2-acetylaminofluorene (AAF)/partial hepatectomy (PH) of resistant hepatocyte(RH) model.Materials & Methods: Through gastrostomy tube, 2-AAF (10㎎/㎏)was administrated daily for 10 days in SD rats. Seventy percent PH was preformed at 5th day of 2-AAF treatment. Animals were killed 1,3,5,7,9 and 14 days following the last day of 2-AAF. Bromodeoxyuridine (BrdU) (100㎎/㎏) was given intraperitoneally 1 hour before killing in each experiment. Hematoxylin eosin-stained histological sections and morphometric changes were analysed by microscope (Americal Optical microStar) with right angle section of portal regions by means of unit areas of high power view. For BrdU incorporation, the sections were immunostained by an anti-BrdU antibody and labelled nuclei were counted. For electron microscopy, fresh liver tissues fixed with 2% glutaraldehyde were processed by routine method. Results:1) It was confirmed that periportal OV cells are a major component of AAF/PH-induced hepatic regeneration based on OV cell proliferation in concord with restoration of liver weight. 2) With BrdU incorporation, OV cell proliferative activity migrates toward pericentral zone from periportal zone. 3) OV cells show bile ductular differentiation with progressive regression and later hepatocytic differentiation through basophilic small hepatocytic transition. 4) In contrast to OV cells, basophilic small hepatocytic emerged from periphery of OV cell proliferative zone in late period of regeneration, inspite of independent pericentral localization of small proportion. 5) Ultrastructurally 3 type of OV cells, at least, were noted in peripotral proliferative zone: predominant biliary differentiated cells, sparce undifferentiated small cells and hepatocytic hepatocytic transitional cells. 6) Colonized hematopoietic cells with a few blast-like cells were noted following OV cell proliferation chiefly in areas of periphery of OV cell proliferative zone. Conclusions: It was confirmed that AAF/PH is a hepatic regeneration model through OV cell proliferation. In this model, hepatic regeneration could be accomplished with periportal OV cell proliferation and subsequent hepatocytic differentiation via small basophilic hepatocytic transition, in addition to a possible role of extramedullary hematopoietic cells.

흉곽내 갑상선종에서 발생한 유두암

이봉근,조성래,조봉균,김종인,이해영,장희경 고신대학교 의학부 2002 高神大學校 醫學部 論文集 Vol.17 No.1

Intrathoracic goiter may be defined as any thyroid enlargement that has the greatest diameter of the its mass by roentgenography inferior to the upper aperture of the thoracic cage made by the sternum, rib, and vertebral bodies. Thyroid carcinoma can be occurred in the intrathoracic goiter, but it is very rare, the incidence of which is 0.3-1.5% of thyroid goiter. The cell type is a follicular carcinoma mostly, and a papillary carcinoma is only 18% of thyroid carcinoma in intrathoracic goiter. Recently, we experienced a case of thyroid papillary carcinoma in intrathoracic goiter in 73-year old female which was removed through cervical incision and partial sternotomy without any complications. The postoperative courses were uneventful.

대장의 염증을 동반한 편충감염 1예

정효진,채종인,최월용,김경민,김종한,배용목,허정호,최영식 고신대학교 의학부 2004 高神大學校 醫學部 論文集 Vol.19 No.1

Trichuris trichiura(whipworm) thrives chiefly in warm, moist tropical countries, but is found throughout the world. The worm derives its name from its characteristic whiplike shape. Most patients are asymptomatic, but only patients with heavy infections become symptomatic. Symptoms of whipworm infection can include diarrhea, dysentery, abdominal pain, vomiting, anemia, weight loss, anorexia, and rectal prolapse. The diagnosis depends on finding Trichuris eggs on stool concentration tests, or by identification of the adult worms, usually by colonoscopy. We report a case of whipworm infection with colonic inflammation that was diagnosed by colonoscopy and was removed by forcep. Colonoscopy is available in the diagnosis and treatment of whipworm infection.

뇌성 마비 청소년의 자아개념

이명화,홍희숙 고신대학교 의학부 2002 高神大學校 醫學部 論文集 Vol.17 No.1

Background/Objective The purpose of this study was to investigate the self-concept and related factors of the cerebral palsied adolescents and to gain the baseline data for development of effective rehabilitation nursing intervention program of the cerebral palsied adolescents. The design of this study was a descriptive survey study. Methods The subjects of the study were 160 cerebral palsied adolescents attending at special schools located in Seoul and Kyonggi and rehabilitation centers located in Seoul, Kyonggi and Kyongnam province. The data was collected to interview with questionaires with school nurse and research assistant understanding the purpose of this study and researcher from May 20 to July 20. 2000. The instrument used for this study was the self-concept scale(50items 4 point scale). Self-Concept Scale had developed by Fitt(1965), which was standardized by Chung(1968) and modified by Kim(1984). Result The data was analyzed by the SPSS/PC+ program using frequency, percentage, mean, standard deviation, t-test, ANOVA & Scheffe test. The results of this study were as follows; 1. The mean score of self-concept was 138.55±17.20(range: 50-200). 2. The subarea score of internal self concept was the highest score in self-identity score (49.39±7.61) and the lowest score in self-satisfaction score (43.93±6.47). 3. The subarea score of external self concept was the highest score in family self (30.19±5.40) and the lowest score in physical self (25,20±4.21). 4. There were statistically significant different in self-identity score according to the age (F=4.466, P=0.13), the grades (F=5.932, P=.005) and occupational training (F=2.49, P=0.4). 5. There were statistically significant different in self-satisfaction score (F=2.281, P=.0491) and self-behavior score (F=3.741 P=.003) according to types of cerebral palsy. 6. There were statistically significant difference in moral ethical self-score according to the grades (F=3.847, P=.023) and types of cerebral palsy (F=2.605, P=.027). 7. There were statistically significant in family self-score according to the ages (F=6.038, P=.003), the grades (F=3.614, P=.029) and occupational training(F=2.010, P=.010). 8. There were statistically significant in family self-score according to the type of cerebral plasy. (F=3.861, P=.003). Conclusion The self-concept of cerebral plasied adolescents was relatively low and related factor of self-concept were age, the grades, occupational training, types of cerebral polsy in cerebral palsied adolescents.

생후 35일부터 호흡곤란을 보였던 선천성 성대하 혈관종 1례

오지은,노은석,박재선,이강대,김영옥,정미희 고신대학교 의학부 2005 高神大學校 醫學部 論文集 Vol.20 No.1

We report a case of subglottic hemangioma in an 40 days old male infant. Since 35th days of birth, he developed sudden onset of dyspnea, hoarseness, and biphasic stridors. Stridors were more prominent in inspiratory phase than expiratory phase, and were not relieved by prone positioning. Fiberscope of vocal cord was tried, but unsuccessful to make any diagnosis because of the extreme severity of the respiratory distress during the examination. Steroids were maintained for 7 days, but there was no symptomatic improvement. Emergent tracheotmoy was done as signs of air hunger were aggravated in spite of oxygen supply. Neck axial CT study was possible only after tracheostomy, and it showed a small caliber trachea which was displaced to the left side. Sagittal reconstruction neck CT scan showed no connection in the upper airway. Diagnostic approch with direct laryngoscope was made in the operating room which revealed round mass located posteroinferiorly of left vocal cord. The mass, covered by normal mucosa and 1×1×1 ㎝ in size, was ablated with CO₂ laser. Sections of resected subglottic mass showed lobular proliferation of small vessles which is compatible with hemangioma. After 3 weeks of tracheostomy, decannulation was done successfully without any complication. Follow-up examination of the baby at one year of age showed excellent condition except small hemangioma of 1×1.5cm size on the skin of posterior chest wall, which was newly developed at age of 3 months.

Induction of Heme Oxygenase-1 Expression by Rapamycin and Paclitaxel in Smooth Muscle and Endothelial Cells of Human and Rat

Park, Chul Bo,Chung, Hun Taeg,Rhew, Hyun Yul 고신대학교 의학부 2005 高神大學校 醫學部 論文集 Vol.20 No.1

Background: Both rapamycin and paclitaxel are currently being used in drug-eluting stent to block the proliferation of vascular endothelial and smooth muscle cells. Heme oxygenase-1 (HO-1) has also known to be anti-proliferative against vascular cells. In this study, it focused on the role of HO-1 in cytoprotection and antiproliferation of paclirtaxel. Methods: Proliferation of cells and metabolic activity of HO-1 were evaluated with the tetrazolium-based assay. HO-1 protein expression was shown by western bolt analysis of vascular smooth muscle cells (VMSCs), or endothelial cells (ECs), using anti-HO-1 polyclonal antibody from untreated cells and cells exposed to rapamycin and paclitaxel. To determine whether the inhibition of paclitaxel on platelet-derived growth factor (PDGF)-detpendent proliferation may be mediated by its induction of HO-1, we exposed cells to paclitaxel, PDGF and ZnPP, an inhibitor of HO activity. Survival rate was checked to fine the anti-apototic effect of paclitaxel against tumor necrosis factor (TNF)-α-mediated apoptosis, in the presence or abscence of ZnPP. Result: Rapamycin induces HO-1 protein in rat VSMCs and human ECs. Exposure of VSMCs to palcitaxel for 12 h resulted in a dose-dependent increase in HO-1 activity. Ten nM of paclitaxel led to a maximal expression of HO-1 protein at 12h after exposure. The addition of Znp abrogated the suppression effect of paclitaxel on PDGF-stimulated cell proliferation, and blocked paclitaxel-mediated suppression of TNF-α mediated apoptosis. Hemoglobin, a scavenger of CO, abrogated the paclitaxel induced cytoprotection. Conclusion: Paclitaxel induced the expression of HO-1 gene in rat VSMCs and human ECs, in dose-dependent and time dependent manner, like rapamycin.